Ichthyosis linearis circumflexa Comèl and Netherton's syndrome; an ultrastructural study.

An electron microscopic study of 3 cases of ichthyosis linearis circumflexa Comèl (ILC) reveals the presence of round bodies in the prickle cells: a characteristic feature of ILC. The horny and the granular layers are absent and are replaced by parakeratotic cells with condensed cytoplasm, which an amorphous material dissociates. The early formation of a vesiculopustule is detected. The importance of the dermal inflammation is considered and it is stated that ILC is not a purely epidermal disorder of keratinization.